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Abstract

  1. Top of page
  2. Abstract
  3. Case Report
  4. Discussion
  5. Acknowledgment
  6. References

Abstract:  Congenital multiple clustered dermatofibroma (MCDF) is a rare, idiopathic, benign tumor presenting at birth as an asymptomatic hyperpigmented patch that is stable until puberty, at which time it enlarges and develops papules. Ultimately, MCDF appears to follow a stable, benign course. We present a case of a 12-year-old girl with congenital MCDF. To our knowledge, this is only the third reported case of congenital presentation of MCDF and the only case featuring atrophoderma-like depression.


Case Report

  1. Top of page
  2. Abstract
  3. Case Report
  4. Discussion
  5. Acknowledgment
  6. References

A healthy 12-year-old girl presented with an asymptomatic hyperpigmented plaque on her lower back. Her parents had noted a subtle, tan, congenital patch that gradually became darker. Superimposed brown macules and papules developed at approximately age 11.

On examination, there was an 8- × 11-cm nontender, irregularly depressed, hyperpigmented plaque on the lower back with overlying dark brown macules and firm papules ranging in size from 2 to 12 mm (Fig. 1). Dermoscopy of a papule revealed a central white area surrounded by a subtle pigment network, suggestive of dermatofibroma.

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Figure 1.  Multiple clustered brown macules and papules overlying a depressed tan plaque on the lower back.

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Histologic examination of biopsies of the atrophic background plaque, the macules, and the papules were identical and consistent with a dermatofibroma. Hematoxylin and eosin staining revealed epidermal hyperplasia with epidermal hyperpigmentation overlying a proliferation of spindled cells arranged in a vague fascicular pattern with collagen trapping. The spindle cells were Factor 13A+, CD34, and actin negative (Fig. 2).

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Figure 2.  Biopsy showing a spindle cell proliferation with collagen trapping (A). CD-34 immunohistochemistry highlights the vascular pattern (40× magnification) (B).

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Discussion

  1. Top of page
  2. Abstract
  3. Case Report
  4. Discussion
  5. Acknowledgment
  6. References

Multiple clustered dermatofibroma is a rare, idiopathic, benign tumor that usually develops during adolescence. The term MCDF is misleading, suggesting the mere grouping of dermatofibromas, when MCDF is a plaque-like proliferation. To our knowledge, this patient represents the fourteenth reported case of MCDF (1) and the third case with congenital onset (2,3).

Multiple clustered dermatofibroma has a predilection for the lower extremities. All reported cases of congenital MCDF have been asymptomatic and stable until puberty, when they entered a growth phase, increasing in size and developing new papules. The atrophoderma-like morphologic feature has not been previously described.

Prognosis of congenital MCDF appears to be good. The grouped papules typically erupt over several years and then stabilize, followed by a benign, asymptomatic course (2). There have been no reports of malignant transformation in MCDF, with follow-up periods ranging up to 20 years (4).

The differential diagnosis of MCDF includes dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyoma, medallion-like hamartoma, and plaque-like myofibroblastic tumor of infancy, which is a recently described actin-positive benign tumor with a clinical appearance to similar that of MCDF (5).

There is no effective treatment other than surgical excision, but excision is often impractical because of the large size, and recurrence has been observed after excision (2). Finally, although congenital MCDF appears to be benign, long-term follow-up is prudent given the rarity of this entity.

Acknowledgment

  1. Top of page
  2. Abstract
  3. Case Report
  4. Discussion
  5. Acknowledgment
  6. References

The authors would like to thank Drs. Ilona Frieden, Andrea Zaenglein, and Ann Marqueling for their insight regarding plaque-like myofibroblastic tumor of infancy.

References

  1. Top of page
  2. Abstract
  3. Case Report
  4. Discussion
  5. Acknowledgment
  6. References